PSYCHOPATHOLOGICAL MANIFESTATIONS OF ACUT E HEPATIC PORPHYRIA

The acute hepatic porphyria is a rare inherited autosomal dominant dis order of heme biosynthesis. It is caused by an enzyme defect of uropor phyrinogen-l-synthestasis. The congenital disease is more often in fem ale patients with a total prevalence of 5-10/100000. Medical and neuro logic symptoms are common and porphyria is initially often misdiagnose d. Psychiatric symptoms are depression, psychoorganic syndromes and sc hizophrenic psychosis. In this article we describe psychopathological symptoms reported in the literature and present a case of porphyria wi th various psychopathological manifestations posing difficulties in di fferentiating from other diagnosis.